Cases reported "Sickle Cell Trait"

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1/8. Successful free flap transfer and salvage in sickle cell trait.

    The presence of sickle cell haemoglobin is generally regarded as a contraindication to free tissue transfer. We present the case of a 42-year-old male with sickle cell trait who had free transfer of a latissimus dorsi flap to cover a gunshot wound to his thigh. His initial haemoglobin S was 36%. Early flap failure from venous thrombosis was successfully salvaged by re-anastomosis to alternative vessels.
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2/8. Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.

    A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.
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3/8. Sudden death due to a haemoglobin variant.

    A previous healthy 35-year-old man was found dead in his truck. Shortly before death he merely complained of influenza-like symptoms. The histological examination revealed evidence of a massive accumulation of sickle cells in smaller blood vessels. After molecular genetic analysis, the preliminary diagnosis of "sickle cell disease" was finally changed to the diagnosis of a sickle cell trait. It is presumed that an epileptic attack which also has to be considered as a concurring cause of death, precipitated sickling of the erythrocytes and led to a fatal sickle cell crisis.
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4/8. Optociliary shunts and sickle retinopathy in a woman with sickle cell trait.

    We report a case of unilateral optociliary shunt vessels and sickle cell retinopathy in a patient with sickle cell trait. Sickle cell retinopathy has rarely been reported in patients with sickle cell trait hemoglobinopathy. To our knowledge, this is the first report of the association of sickle cell trait, unilateral sickle cell retinopathy, and ipsilateral optociliary shunt vessels.
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5/8. hypertension in sickle cell disease.

    We report a young male with sickle cell trait who developed severe hypertension and splenic infarction soon after travel to a high altitude. hypertension persisted for three days after a diagnostic laparotomy. His blood pressure then continued to be normal over the next one and a half years. Red cell sludging in the small vessels of the kidney possibly activated the renin angiotensin system.
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6/8. Iatrogenic choroidal neovascularization in sickle cell retinopathy.

    Fifteen patients (16 eyes) with proliferative sickle retinopathy treated with argon or xenon arc feeder vessel photocoagulation had chorioretinal (CRN) or choriovitreal (CVN) neovascularization develop. These patients were followed from 2 1/2 to 11 1/2 years with a mean follow-up of 6 years and 8 months. Clinically important late complications of the choroidal neovascularization included vitreous hemorrhage (in three of eight patients with CVN). However, in only two of these three eyes was there any drop in vision related to residual vitreous hemorrhage, and this was limited to loss of only one line of Snellen visual acuity. Therefore, treatment is not usually recommended if CRN or CVN develops after intense photocoagulation. Vitreous fluorophotometry was performed on these patients to examine the breakdown of the blood-retinal barrier. This gave a quantitative measure of fluorescein leakage not obtained with fluorescein angiography. Midvitreous measurements, which are more representative of these peripherally located proliferative lesions than are pre-retinal measurements, suggest that vitreous fluorophotometry may be helpful in differentiating the higher leakage of CVN from the CRN.
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7/8. myocardial revascularization in a sickle-cell trait patient.

    A 56-year-old man with confirmed sickle-cell trait AS underwent successful triple-vessel myocardial revascularization with internal mammary artery and vein conduits. Variations in routine technique included limitation in hypothermia (32 degrees C), avoidance of intraoperative autologous salvage, hypoxia, severe anemia (< 25% hematocrit), acidosis and postoperative reinfusion of mediastinal drainage.
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8/8. Intracranial aneurysms and sickle cell anemia: multiplicity and propensity for the vertebrobasilar territory.

    OBJECTIVE: We describe a case of sickle cell anemia and multiple intracranial aneurysms and review the English-language-reported cases of sickle cell disease associated with intracranial aneurysms proven angiographically or by autopsy, to assess whether there are associations with aneurysm multiplicity and sites of aneurysm occurrence. CLINICAL PRESENTATION: A 28-year-old woman with sickle cell disease and a subarachnoid hemorrhage underwent successful clipping of three intracranial aneurysms. RESULTS: Among 44 reviewed cases, 57% of patients demonstrated multiple aneurysms, and aneurysms from patients with multiple aneurysms comprised nearly 80% of the total number of aneurysms. There were, on average, three aneurysms per patient for patients with multiple aneurysms. There was a predominance of female patients (female/male ratio, 1.6:1), although there existed no significant differences in age or gender for patients with single or multiple aneurysms. None of the patients with multiple aneurysms was older than 40 years of age at the time of presentation. patients with multiple aneurysms and sickle cell disease showed a significant difference in the distribution of the aneurysm sites, with a significantly large number occurring in the vertebrobasilar axis. Multiple aneurysms associated with sickle cell disease showed a higher rate of simultaneous occurrence in the posterior and anterior circulation, compared with multiple aneurysms in the general population. CONCLUSION: There are strong statistical associations for aneurysm multiplicity and sites of aneurysm occurrence among reported patients with sickle cell disease. patients with sickle cell anemia and neurological symptoms should undergo magnetic resonance angiography or four-vessel angiography to detect potentially harmful, but neurosurgically treatable, pathological conditions.
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